Nephrotic Syndrome

Nephrotic Syndrome:

 Nephrotic syndrome is a collection of symptoms that cause kidney damage.  It contains protein in the urine, low blood albumin levels, high blood lipids, and significant inflammation. Other symptoms may include weight gain, feeling tired, and foaming. Complications can include blood clots, infections, and high blood pressure. 

Causes include many kidney diseases such as focal segmental glomerulosclerosis, membrane nephropathy, and minimally invasive disease.  It can also be a complication of diabetes or lupus.  The underlying mechanism usually damages the glomeruli of the kidneys. Diagnosis is usually based on a urine test and sometimes a kidney biopsy.  This is different from nephritic syndrome because there are no red blood cells in the urine. 

Treatment is directed at the underlying cause.  Other efforts include high blood pressure management, high blood cholesterol, and infection risk.  Low-salt diets and limited fluids are often recommended. About 100,000 people are affected each year.  The common underlying cause varies between children and adults.

Sign and Symptoms:

Nephrotic syndrome is characterized by severe proteinuria (> 3.5. G per 1.73 m2 body surface area,  or> mg0 mg per square meter body surface area per hour in children), hypoalbuminemia (<2.0 g / dl). , hyperlipidaemia, and edema begin on the face. Lipiduria (lipids in the urine) may also appear, but is not necessary for the diagnosis of nephrotic syndrome. Hyponatremia also occurs with a low partial sodium excretion.

Hyperlipidemia is caused by two causes: 

Hypoproteinemia stimulates protein synthesis in the liver, resulting in excessive production of lipoproteins.

Lipid catabolism is reduced due to low levels of lipoprotein lipase, the main enzyme involved in lipoprotein breakdown.  Cofactors, such as apolipoprotein C2, can also be lost from increased filtration of proteins.

Some of the other features seen in nephrotic syndrome are:

The most common symptom is excess fluid in the body due to serum hypoalbuminemia. Lower serum oncotic pressure causes fluid to accumulate in the interstitial tissues. Sodium and water retention stimulates edema. It can take many forms:

Eyes swollen around the eyes in the morning.

Pitting edema on the legs.

Lung flow due to fluid in the lung cavity. More fluid is often associated with pulmonary edema.

Ascites is caused by fluid in the peritoneal cavity.

In the normal body, edema is known as ansar.

Most people with nephrotic syndrome are normal, but high blood pressure (rarely) may occur.

Anemia (iron-resistant microcritical hypochromic type) may be due to transferrin deficiency.

Dyspnea can be caused by furrow outflow or by diaphragmatic compression with ascites.

Erythrocyte sedimentation rate is increased due to increased fibrinogen and other plasma content.

Some people may see foam or fruit urine because of severe proteinuria, which reduces surface tension. Real occupational complaints such as hematuria or oliguria are rare, although they are commonly seen in nephritic syndrome.

There may be features of the underlying cause, such as systemic lupus erythematosus, or chronic diabetes-related neuropathy.

The examination should not include other causes of gross edema, especially the cardiovascular and liver systems.

Muehrcke not nails; The white line (Leukonichia) that extends all the way across the fort and parallels the Lunula 

The main symptoms of nephrotic syndrome are: 

Proteinuria in the nephrotic range between 3.5 g / 24h / 1.73 m2 (between 3 and 3.5 g  / 24 h  / 1.73  m2 is consider to be proteinuria in nephrotic range) or proteinuria greater than 40 mg / h / m2 in children.  The ratio of albumin to creatinine urinary secretion can be used for total protein in the absence of a 24-hour urine test. This coefficient will be greater than 200–00 mg / mmol in nephrotic syndrome. This apparent loss of protein is due to an increase in glomerular permeability which allows proteins to pass into the urine instead of into the blood. Under normal circumstances, a 24-hour urine sample should not exceed 1 milligram or 10 milligrams per liter. 

A hypoalbuminemia of less than 2.0 g / dL,  which is higher than the clearance level of the liver, i.e., protein synthesis in the liver is insufficient to increase the level of low blood protein.

Edema is thought to be caused by two mechanisms. The first is hypoalbuminemia which reduces the oncotic pressure inside the vessels and results in hypovolemia and activation of the renin-angiotensin system and thus maintaining sodium and water. Additionally, albumin is thought to have a direct effect on the epithelial sodium channel  in the principal cell leading to reabsorption of sodium and water. Nephrotic syndrome initially appears in the lower body parts (such as the legs) and in the eyelids. In advanced stages it extends to the cavernous cavity and peritoneum (hydrocephalus) and may even develop as a generalized anastomosis.

Hyperlipidaemia is caused by an increase in the synthesis of low and very low density lipoproteins in the liver which is responsible for the transport of cholesterol and triglycerides. Cholesterol has also been shown to increase liver synthesis.

Thrombophilia, or hypercoagulability, is a major outbreak for the formation of blood clots that are lost in the urine due to a decrease in the level of antithrombin III in the blood.

Lipiduria or lipid deficiency in urine is an indicator of glomerular pathology due to increased filtration of lipoproteins. 

Diagnosis:

In addition to obtaining a complete medical history, a series of biochemical tests are required to arrive at the correct diagnosis to prove the presence of the disease. Additionally, kidney imaging (for the structure and appearance of two kidneys) is sometimes performed, and / or kidney biopsy. The first test would be a urinalysis to test for high levels of protein, [22] as healthy subjects excrete a small amount of protein in their urine. The test involves a total urine protein estimate in a 24-hour bed. Urine samples are tested for proteinuria (> 3.5. Hours g per 1.7373 m2 per 2 hours). It is also checked for urine cast, which is more characteristic of active nephritis. Another blood screen, Detailed Metabolic Panel (CMP) will detect hypoalbuminemia: albumin level of album2..5 g / dL (normal = -5.-5--5 g / dL). The creatinine clearance CCR test then evaluates the function of the kidneys, especially the glomerular filtration capacity. Creatinine formation is the result of damage to muscle tissue, which is transported into the blood and ends up in the urine. Measuring the concentration of biological compounds in both fluids evaluates the ability of the glomeruli to filter blood. Electrolytes and urea levels can also be analyzed simultaneously as creatinine (EUC test) to assess the value of kidney function. A lipid profile is also excluded as high levels of cholesterol (hypercholesterolemia), especially upper LDL, usually together with upper VLDL, is an indicator of nephrotic syndrome.

A kidney biopsy can also be used as a more specific and invasive test method. The study of the anatomical pathology of the specimen may then allow the identification of the type of glomerulonephritis. [2] However, this procedure is generally reserved for adults because the majority of children suffer from minimally invasive disease with corticosteroids having a  remission rate. Biopsies are usually indicated only for children who are corticosteroid resistant because the majority suffer from focal and segmental glomerulosclerosis. 

Further investigations are indicated if the cause involves analysis of autoimmune markers (ANA, ASOT, C, cryoglobulin, serum electrophoresis), or a full abdominal ultrasound.

Classification

A broad classification of nephrotic syndrome based on the underlying cause:

Nephrotic Syndrome

1.Primary   2.secondary

Nephrotic syndrome is often classified as histologically:

Nephrotic syndrome

1. MCD   2. FSGS   .MGN  4. MPGN

Different diagnoses

Some of the symptoms that occur in nephrotic syndrome, such as edema and proteinuria, also appear in other patients. Therefore, other malformations must be eliminated in order to reach a definitive diagnosis. 

Edema: In addition to nephrotic syndrome, there are two other disorders that often accompany edema; These are heart failure and liver failure.  Congestive heart failure can cause fluid retention in tissues as a result of lack of strength of ventricular contraction. Initially the liquid is concentrated in the knees but later it is generalized and it is called Annasar.  People with congestive heart failure also experience abnormal inflammation of the cardiomegaly, which helps make an accurate diagnosis. Jugular venous pressure can also be raised and heart murmurs can be heard. Echocardiogram is the preferred research method for these symptoms. Liver failure due to cirrhosis, hepatitis and other conditions such as alcohol consumption, IV drug use or some hereditary diseases can lead to inflammation in the lower extremities and abdominal cavity. Other symptoms include jaundice, varicose veins over the umbilicus (thin medusi), scratch marks (due to widespread itching, known as pruritus), enlarged pleura, spider angiomata, encephalopathy, anomalies in nodular liver and liver function tests.  Some of the symptoms associated with repeated drug administration should be relieved. These drugs promote fluid retention such as NSAIDs, some antihypertensive drugs, adrenal corticosteroids and sex hormones. 

Acute fluid overload can lead to edema in someone with kidney failure. These people are known to have kidney failure, and either drink too much or miss their dialysis. Additionally, when metastatic cancer spreads to the lungs or abdomen, it causes blockage of lymphatic vessels and veins, as well as serous exudation, and fluid and circulation infections.

Proteinuria: Protein loss from urination is caused by a number of pathological agents, and these agents have to be denied before it can be confirmed that the infected person has nephrotic syndrome. Multiple myeloma can be a proteinuria that does not accompany hypoalbuminemia, which is an important aid for differential diagnosis;  Other possible causes of proteinuria are also included.